Wal, L. van der (Laurine) (2019) Hypertrophic Cardiomyopathy Imaging in Children : and how it improves risk prediction for sudden cardiac death and adverse events. thesis, Medicine.
Full text available on request.Abstract
Background: Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disorder, with a population prevalence of 1 in 500 in adults. The majority of patients with HCM are asymptomatic. Nevertheless, patients are at risk for catastrophic events, including sudden cardiac death (SCD). The aims of this study were: 1) to quantify the parameters of left ventricle (LV) size, function, and structural abnormalities, and 2) To assess for associations between maximum LV wall thickness, LV function parameters, risk factors for SCD and adverse events at the time of Cardiovascular Magnetic Resonance (CMR) and during follow-up. Methods: The study was a multicentre retrospective cohort study. Patients <21 years of age with HCM who underwent a CMR study were used to quantify myocardial parameters. Additionally, the clinical information was collected. Results: CMR exams were performed on 559 HCM patients (13.8±4.2years; 75% males) in 34 pediatric centers. Average maximum LV wall thickness was 12.9±7.0mm/m2 which was associated with the appearance of ≥1 risk factor for SCD. SCD risk factors are associated with adverse events with each additional risk factor the OR increased by 2.66. SCD occurred in 3.8%, of which all were asymptomatic or minimally asymptomatic. Structural abnormalities were frequently seen (crypts 24%, apical-basal muscle bundles 27%). Conclusion: Myocardial parameters, such as mass, systolic function and volumes in not only the LV, but also in right ventricle, are abnormal in children and adolescents with HCM. The resulting alterations in myocardial parameters are associated with an increase in risk factors for SCD and heart failure. Besides the myocardial parameters, SCD risk factors are an important tool to predict for SCD events, although most patients who died of SCD were asymptomatic. Finally, structural abnormalities were seen frequently, although their clinical significance stays unknown
| Item Type: | Thesis (UNSPECIFIED) |
|---|---|
| Supervisor name: | Grosse-Wortmann, L. and Marchie Sarvaas, G.J. du |
| Faculty: | Medical Sciences |
| Date Deposited: | 13 Oct 2020 10:42 |
| Last Modified: | 13 Oct 2020 10:42 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/2774 |
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